Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: An Observational Cohort Study

Abstract BACKGROUND: Cauda equina hemangioblastomas in von Hippel-Lindau (VHL) disease can cause significant neurological signs and symptoms. Despite their associated morbidity, the management of these tumors remains incompletely defined. OBJECTIVE: To determine optimal management, we analyzed the functional outcomes after resection of these tumors. METHODS: VHL patients who underwent surgical resection of cauda equina hemangioblastomas at the National Institutes of Health and the University of Virginia were included. Clinical and radiological follow-up was performed at 6- to 12-month intervals after surgery. RESULTS: Fifteen patients underwent 18 operations for 21 cauda equina hemangioblastomas (median follow-up 5.9 years). Patients often presented with multiple symptoms, including pain (67%), numbness (50%), urinary complaints (33%), and weakness (11%). Median preoperative tumor volume was 1.2 cm3. Four tumors at 3 operations were not resected due to a motor nerve root origin. Gross total resection was achieved in 14 surgeries (93% of operations when resection was attempted). New mild (non-function limiting) neurological symptoms were noted after 11 operations (61%), which most often (64%) resolved within 2 weeks of surgery. At 6-month follow-up, 15 patients (83%) were stable, 2 (11%) were improved, and 1 (6%) was worse. Histological analysis revealed that all tumors originated from within the involved nerve fascicle. CONCLUSIONS: VHL-associated cauda equina hemangioblastomas have an intrafascicular origin and require interruption of the rootlet of origin for complete resection. Motor nerve root involvement may preclude complete resection but strategies including bony decompression and/or interruption of vascular supply may provide a therapeutic option. Nevertheless, most VHL patients with symptom-producing lesions improve with resection.

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Acute cauda equina syndrome from a ruptured aneurysm in the sacral canal

Journal of Neurosurgery ◽

10.3171/jns.1992.77.6.0945 ◽

1992 ◽

Vol 77 (6) ◽

pp. 945-948 ◽

Cited By ~ 9

Author(s):

Richard H. Schmidt ◽

M. Sean Grady ◽

Wendy Cohen ◽

Sanford Wright ◽

H. Richard Winn

Keyword(s):

Cauda Equina Syndrome ◽

Cauda Equina ◽

Signs And Symptoms ◽

Vascular Supply ◽

Ruptured Aneurysm ◽

Collateral Flow ◽

Content Type ◽

Internal Iliac ◽

The Right ◽

Sacral Canal

✓ The case is presented of a young woman with acute cauda equina syndrome from a ruptured aneurysm in the sacral canal. The lesion was associated with pathological enlargement of the lateral sacral arteries bilaterally, which presumably occurred to provide cross-pelvic collateral flow in response to the diversion of the right internal iliac artery for renal transplantation. The patient presented with signs and symptoms of spontaneous spinal epidural hemorrhage. The radiographic features of this lesion are described. In addition to angiography and partial embolization of the vascular supply, contrast-enhanced high-resolution computerized tomography was essential in the diagnosis and treatment of this unique aneurysm.

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Microsurgical resection of cauda equina schwannoma with nerve root preservation

Neurosurgical FOCUS ◽

10.3171/2014.v3.focus14267 ◽

2014 ◽

Vol 37 (v2supplement) ◽

pp. Video6

Author(s):

Paul C. McCormick

Keyword(s):

Nerve Root ◽

Motor Nerve ◽

Cauda Equina ◽

Sensory Nerve ◽

Motor Deficit ◽

Link Type ◽

Microsurgical Resection ◽

Spinal Schwannoma

The occurrence of motor deficit following resection of an intradural spinal schwannoma is an uncommon but potentially serious complication. This video illustrates the technique of microsurgical resection of an L-4 sensory nerve root schwannoma with preservation of the corresponding functional L-4 motor nerve root.The video can be found here: http://youtu.be/HrZkGj1JKd4.

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Early outcomes of conformal radiotherapy in the treatment of cavernous sinus meningioma

Journal of Radiotherapy in Practice ◽

10.1017/s1460396918000535 ◽

2018 ◽

Vol 18 (02) ◽

pp. 150-154

Author(s):

R. Moujahed ◽

K. Ghedira ◽

C. Drissi ◽

A. Kallel ◽

L. Kochbati

Keyword(s):

Cavernous Sinus ◽

Tumour Progression ◽

Therapeutic Option ◽

Tumour Size ◽

Signs And Symptoms ◽

Conformal Radiotherapy ◽

Lesion Size ◽

Conservative Strategy ◽

Cavernous Sinus Meningioma

Abstract Aim: Cavernous sinus tumours represent 1% of all intracranial neoplasms, and 41% of them are cavernous sinus meningiomas (CSM). The disappointing results of the microsurgical approach in the treatment of CSM have led to the evaluation of a more conservative strategy, such as conformal radiotherapy (CR) and stereotactic radiosurgery (SRS). Here we report our experience with CR in the treatment of CSM, aiming to evaluate local control, clinical response and radiation-induced toxicity. Methods: A total of 18 patients with CSM, treated from 2011 to 2017, were retrospectively reviewed. Results: Patient median age was 59 years [31–74]. Clinical presentation included impaired vision, cranial nerve deficit, headache, ocular signs. Median tumour size was 35 mm [13–56]. Six patients were operated before the radiation. Twelve patients were treated with definitive radiotherapy. Dose of radiation varied from 50.4 Gy in 28 fractions, to 54 Gy in 30 fractions. Median follow-up was 33 months [6–84]. Among the 18 patients, patient’s signs and symptoms of disease remained unchanged in three cases (16.66%), 12 (66.66%) were improved and 1 patient (5.55%) exhibited tumour progression (exophthalmia). Two patients (11.11%) were lost to follow-up after the end of the treatment with radiotherapy. Findings: Based on our study findings, CR has proved to be a relatively safe and effective therapeutic option in the treatment of CSM, whether used as first-line or as an adjuvant treatment. CR may be particularly effective for tumours that are not amenable to SRS, owing to lesion size (> 30-35 mm) and/or proximity to the optic apparatus (<3 mm).

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Sacral hemangioblastoma in a patient with von Hippel–Lindau disease

Neurosurgical FOCUS ◽

10.3171/foc.2003.15.2.11 ◽

2003 ◽

Vol 15 (2) ◽

pp. 1-4 ◽

Cited By ~ 13

Author(s):

Ryszard M. Pluta ◽

Scott D. Wait ◽

John A. Butman ◽

Kathleen A. Leppig ◽

Alexander O. Vortmeyer ◽

...

Keyword(s):

Nerve Root ◽

Histopathological Diagnosis ◽

Resonance Imaging ◽

Nerve Roots ◽

Von Hippel Lindau ◽

Sacral Nerve ◽

Left Lower Extremity ◽

History Of ◽

Von Hippel Lindau Disease

Hemangioblastomas are histologically benign neoplasms that occur sporadically or as part of von Hippel–Lindau disease. Hemangioblastomas may occur anywhere along the neuraxis, but sacral hemangioblastomas are extremely rare. To identify features that will help guide the operative and clinical management of these lesions, the authors describe the management of a large von Hippel–Lindau disease–associated sacral hemangioblastoma and review the literature. The authors present the case of a 38-year-old woman with von Hippel–Lindau disease and a 10-year history of progressive back pain, as well as left lower-extremity pain and numbness. Neurological examination revealed decreased sensation in the left S-1 and S-2 dermatomes. Magnetic resonance imaging demonstrated a large enhancing lesion in the sacral region, with associated erosion of the sacrum. The patient underwent arteriography and embolization of the tumor and then resection. The histopathological diagnosis was consistent with hemangioblastoma and showed intrafascicular tumor infiltration of the S-2 nerve root. At 1-year follow-up examination, pain had resolved and numbness improved. Sacral nerve root hemangioblastomas may be safely removed in most patients, resulting in stabilization or improvement in symptomatology. Generally, hemangioblastomas of the sacral nerve roots should be removed when they cause symptoms. Because they originate from the nerve root, the nerve root from which the hemangioblastoma originates must be sacrificed to achieve complete resection.

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P.037 Disseminated leptomeningeal hemangioblastoma in a case of Von Hippel Lindau

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2018.139 ◽

2018 ◽

Vol 45 (s2) ◽

pp. S25-S26

Author(s):

FB Maroun ◽

N Hache ◽

P Bartlett ◽

B Galway ◽

A Engelbrecht ◽

...

Keyword(s):

Cauda Equina ◽

Leptomeningeal Dissemination ◽

Review Of The Literature ◽

Shunt Insertion ◽

Von Hippel Lindau ◽

Suprasellar Region ◽

Subarachnoid Spaces ◽

Ambient Cistern

Background: Leptomeningeal dissemination of hemangioblastomas (HB), whether sporadic or associated with Von Hippel Lindau (VHL), are extremely rare. Very scanty literature is available. Methods: A 36 year old female with VHL and stable pancreatic, adrenal and renal lesions was operated upon 4 years ago for a large symptomatic cervicomedullary cystic and solid tumor. 2 years after surgery the tumour recurred and further removal was unsuccessful due to medullary adhesions. Radiation was given to the posterior fossa area and to several small nodules over the cauda equina resulting in severe pain. Serial follow up imaging revealed diffuse leptomeningeal dissemination increasing in size of the suprasellar region, ambient cistern and Sylvian fissures. Clinically, she has been stable with small dose of steroids and VP shunt insertion for papilledema. Results: Review of the literature consists of 2 series of 7 and 21 patients each with leptomeningeal dissemination involving sporadic HB and VHL associated HB. Leptomeningeal dissemination is estimated at about 4.3%. It is postulated that the tumour starts in the Pia and spreads in an extra medullary fashion throughout the subarachnoid spaces. Conclusions: Long term recurrence has been noted raising the question of aggressive treatment with some drug therapy related to angiogenesis is postulated.

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Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease

Journal of Neurosurgery ◽

10.3171/2014.1.jns131431 ◽

2014 ◽

Vol 120 (5) ◽

pp. 1055-1062 ◽

Cited By ~ 82

Author(s):

Russell R. Lonser ◽

John A. Butman ◽

Kristin Huntoon ◽

Ashok R. Asthagiri ◽

Tianxia Wu ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Natural History ◽

Cauda Equina ◽

Area Under The Curve ◽

Neurological Impairment ◽

Clinical Trial Registration ◽

Von Hippel Lindau ◽

Male Sex

Object The tumors most frequently associated with von Hippel-Lindau (VHL) disease are hemangioblastomas. While they are associated with significant neurological impairment and mortality, their natural history and optimal management have not been fully defined. Methods Patients with VHL were enrolled in a prospective study designed to define the natural history of CNS hemangioblastomas. In the present analysis, serial imaging, laboratory, genetic, and clinical data were evaluated in those with at least 2 years of follow-up data. Results At study entrance 225 patients (111 males, 114 females) harbored 1921 CNS hemangioblastomas in the supratentorial compartment (21 tumors [1%]), cerebellum (865 [45%]), brainstem (129 [7%]), spinal cord (689 [36%]), cauda equina (212 [11%]), and nerve roots (5 [0.3%]; follow-up 15,819 hemangioblastoma-years). Increased tumor burden was associated with partial deletions in the VHL gene (p = 0.005) and male sex (p = 0.002). Hemangioblastoma development (median 0.3 new tumors/year) was associated with younger age (p < 0.0001) and more tumors at study entrance (p < 0.0001). While 1278 hemangioblastomas (51%) did not grow, 1227 hemangioblastomas (49%) grew in a saltatory (886 [72%]), linear (76 [6%]), or exponential (264 [22%]) pattern. Faster tumor growth was associated with male sex (p = 0.001), symptomatic tumors (p < 0.0001), and tumors associated with cysts (p < 0.0001). Location-dependent tumor size was the primary predictor of eventual symptom formation (159 symptomatic tumors [6.3%]; area under the curve > 0.9). Conclusions Central nervous system hemangioblastoma burden in VHL is associated with partial germline deletions and male sex. Unpredictable growth of hemangioblastomas compromises assessment of nonsurgical therapies. The judicious treatment of symptom-producing hemangioblastomas, while avoiding unnecessary treatment of asymptomatic tumors that may not progress, can provide clinical stability. Clinical trial registration no.: NCT00005902 (ClinicalTrials.gov).

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Full-Endoscopic Lumbar Decompression with Minimal Nerve Root Retraction for Impending Neurologic Deficit in Degenerative Lumbar Spine Diseases

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0041-1725955 ◽

2021 ◽

Author(s):

Hyeun Sung Kim ◽

Harshavardhan Dilip Raorane ◽

Il Choi ◽

Pang Hung Wu ◽

Kyung Hoon Yang ◽

...

Keyword(s):

Lumbar Spine ◽

Spine Surgery ◽

Nerve Root ◽

Cauda Equina ◽

Neurologic Deficit ◽

Foot Drop ◽

Degenerative Lumbar Spine ◽

Endoscopic Spine Surgery ◽

Interlaminar Approach

Abstract Objectives The aim of this retrospective case study was to analyze the outcomes of minimal nerve root retraction in patients with impending neurologic deficit in degenerative lumbar spine disease using the full-endoscopic spine surgery. Materials and Methods Thirty-seven consecutive patients with impending neurologic deficit underwent endoscopic spine surgery through either the transforaminal or the interlaminar approach. Their clinical outcomes were evaluated with visual analog scale (VAS) leg pain score, Oswestry Disability Index (ODI), and MacNab's criteria. The outcome of motor deficitis was evaluated with the Medical Research Council (MRC) grade. Completeness of decompression was documented with a postoperative magnetic resonance imaging (MRI) and computed tomography (CT) scan. Results A total of 40 lumbar levels of 37 patients were operated, VAS score of the leg improved from 7.7 ± 1 to 1.9 ± 0.6 (p < 0.0001). ODI score improved from 74.7 ± 6.5 to 25.4 ± 3.49 (p < 0.0001). Motor weakness improved significantly immediately after surgery. The mean MRC grade increased to 1.97, 3.65, 4.41, and 4.76 preoperatively, at 1 week, at 3 months, and at the final follow-up, respectively, and all the patients with foot drop and cauda equina syndrome symptom recovered completely. One patient with great toe drop recovered partially to MRC grade 3. Mean follow-up of the study was 13.3 ± 6.1 months. According to MacNab's criteria, 30 patients (80.1%) had good and 7 patients (18.9%) had excellent results. Three patients required revision surgery. Conclusions Minimal nerve root retraction during full-endoscopic spine surgery is safe and effective for treatment of the impending neurologic deficit. We could achieve a thorough decompression of the affected nerve root with acceptable clinical outcome and minimal postoperative morbidity.

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Glomus Jugulare Tumors

Neurosurgery ◽

10.1227/01.neu.0000228682.92552.ca ◽

2006 ◽

Vol 59 (3) ◽

pp. 561-569 ◽

Cited By ~ 58

Author(s):

Massimo Gerosa ◽

Anna Visca ◽

Paolo Rizzo ◽

Roberto Foroni ◽

Antonio Nicolato ◽

...

Keyword(s):

Tumor Volume ◽

Therapeutic Option ◽

Gamma Knife Radiosurgery ◽

Growth Control ◽

Signs And Symptoms ◽

Endovascular Embolization ◽

Neurological Deficits ◽

Surgical Removal ◽

Glomus Jugulare

Abstract OBJECTIVE: Glomus jugulare tumors are generally considered slow-growing, benign lesions. However, their pronounced local aggressiveness frequently results in severe neurological deficits. Surgical removal is rarely radical and is usually associated with morbidity. There is increasing evidence that stereotactic radiosurgery, particularly gamma knife radiosurgery (GKR), may play a relevant role as a therapeutic option in these tumors. METHODS: Between 1996 and 2005, we used GKR to treat 20 patients bearing growing glomus jugulare tumors, mostly classified as Glasscock-Jackson Grade IV or Fisch Stage D1. Follow-up (mean, 50.85 mo) data was available for 20 patients (four men, 16 women; mean age, 56 yr): eight out of 20 tumors were surgical recurrences, three out of 20 patients had GKR as the primary treatment, and 11 out of 20 patients previously underwent endovascular embolization. Regarding the radiosurgical dose planning, the average tumor volume was 7.03 cm3 (range, 1.5–13.4 cm3) and the mean marginal dose was 17.3 Gy (range, 13–24 Gy). RESULTS: Neurological signs and symptoms were unchanged in 13 out of 20 patients. An improvement of cranial nerve function was observed in five patients and hearing deterioration was observed in two patients. Tumor volume was unchanged in 11 out of 20 patients and was slightly (≤ 20%) decreased in eight out of 20 patients. In one unusual case of a bulky cavernous sinus recurrence, neoplastic regression was particularly pronounced. CONCLUSION: Despite the constraints of the limited case material, considering the estimated doubling time of these rare tumors (4.2 yr), our preliminary results with GKR at a mid-term follow-up examination suggest an effective tumor growth control with negligible incidence of untoward sequelae.

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Hybrid Endovascular Repair of Thoracoabdominal Aorta: Early Results

The Heart Surgery Forum ◽

10.1532/hsf98.2014315 ◽

2014 ◽

Vol 17 (3) ◽

pp. 146

Author(s):

Osman Tansel Darcin ◽

Mehmet Kalender ◽

Ayse Gul Kunt ◽

Okay Guven Karaca ◽

Ata Niyazi Ecevit ◽

...

Keyword(s):

Endovascular Repair ◽

Therapeutic Option ◽

Complete Recovery ◽

Aortic Aneurysms ◽

Thoracoabdominal Aorta ◽

Mortality And Morbidity ◽

Early Results ◽

The Mean ◽

Procedural Success

Background: Thoracoabdominal aortic aneurysms (TAAA) present a significant clinical challenge, as they are complex and require invasive surgery. In an attempt to prevent considerably high mortality and morbidity in open repair, hybrid endovascular repair has been developed by many authors. In this study, we evaluated the early-term results obtained from this procedure.Methods: From November 2010 to February 2013, we performed thoracoabdominal hybrid aortic repair in 18 patients. The mean age was 68 years (12 men, 6 women). All of the patients had significant comorbidities. Follow-up computed tomography (CT) scans were performed at 1 week, 3 months, 6 months, and annually thereafter.Results: All patients were operated on in a staged procedure and stent graft deployment was achieved. Procedural success was achieved in all cases. All patients were discharged with complete recovery. No endoleaks weres detected in further CT examination.Conclusion: Our results suggests that hybrid debranching and endovascular repair of extensive thoracoabdominal aneurysms represents a suitable therapeutic option to reduce the morbidity and mortality of TAAA repair, particularly in those typically considered at high risk for standard repair.

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Microsurgical resection of foramen magnum meningioma through a transcondylar approach: three-dimensional operative video

Neurosurgical Focus: Video ◽

10.3171/2019.10.focusvid.19465 ◽

2019 ◽

Vol 1 (2) ◽

pp. V3

Author(s):

Guilherme H. W. Ceccato ◽

Rodolfo F. M. da Rocha ◽

Duarte N. C. Cândido ◽

Wladimir O. Melo ◽

Marcio S. Rassi ◽

...

Keyword(s):

Nerve Palsy ◽

Three Dimensional ◽

Foramen Magnum ◽

Complete Resection ◽

Neurophysiological Monitoring ◽

Cervical Instability ◽

Transcondylar Approach ◽

Postoperative Mri ◽

Microsurgical Resection

Foramen magnum (FM) meningiomas are challenging lesions. We present the case of a 38-year-old female with neck pain, dysphonia, and slight twelfth nerve palsy. Imaging workup was highly suggestive of an FM meningioma, and microsurgical resection with the aid of intraoperative neurophysiological monitoring was indicated. A transcondylar approach was employed, the vertebral artery was mobilized, and the tumor was completely removed. Postoperative MRI demonstrated complete resection. There were no signs of cervical instability. The patient presented with improvement of her symptoms and no new neurological deficit on follow-up. FM meningiomas can be successfully resected using a transcondylar approach, since it increases the exposure of the ventral FM, allowing the surgeon to work parallel to the skull base and flush with the tumor’s attachment. Informed consent was obtained from the patient for publication of this operative video.The video can be found here: https://youtu.be/itfUOB-6zM0.

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